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ABSITE. > chapter 43: pediatric surgery > Flashcards

chapter 43: pediatric surgery Flashcards

1
Q

embryology: lungs, esophagus, stomach, pancreas liver, gallbladder, bile duct, and duodenum proximal to ampulla

A

foregut

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2
Q

embryology: lungs, esophagus, stomach, pancreas liver, gallbladder, bile duct, and duodenum proximal to ampulla

A

foregut

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3
Q

embryology: duodenum distal to ampulla, small bowel, and large bowel to distal 1/3 of transverse colon

A

midgut

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4
Q

distal 1/3 of transverse colon to anal canal

A

hindgut

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5
Q

how does the midgut develop?

A

midgut rotates 270 degrees counterclockwise normally

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6
Q

low birth weight

A
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7
Q

premature

A
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8
Q

immunity at birth

A

IgA from mother’s milk

IgG crosses the placenta

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9
Q

1 cause of childhood death

A

trauma

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10
Q

trauma bolus

A

20cc/kg x 2, then give blood 10 cc/kg

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11
Q

best indicator of shock

A

tachycardia (neonate > 150 ; 120 ; rest > 100)

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12
Q

trauma: goal urine output

A

> 2-4cc/kg

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13
Q

GFR in children (

A

children (

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14
Q

what causes bone growth in children?

A

increased alkaline phosphatase in children compared with adults

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15
Q

what are the umbilical vessels?

A

2 arteries and 1 vein

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16
Q

maintenance IV fluids

A

4cc/kg/hr for first 10 kg
2cc/kg/hr for 2nd 10 kg
1cc/kg/hr for everything after that

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17
Q

four kinds of congenital cystic disease of the lung

A
  • pulmonary sequestration
  • congenital lobar overinflation
  • congenital cystic adenoid malformation
  • bronchiogenic cyst
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18
Q
  • lung tissue has anomalous systemic arterial supply (thoracic aorta or abdominal aorta through inferior pulmonary ligament)
  • have either systemic venous or pulmonary vein drainage
  • do not communicate with tracheobronchial tree
A

pulmonary sequestration

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19
Q

pulmonary sequestration: more likely to have systemic venous drainage (azygous system)

A

extra-lobar pulmonary sequestration

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20
Q

pulmonary sequestration: more likely to have pulmonary vein drainage

A

intra-lobar pulmonary sequestration

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21
Q

tx: pulmonary sequestration (congenital cystic disease of the lung)

A

lobectomy

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22
Q

tx: pulmonary sequestration (congenital cystic disease of the lung)

A

lobectomy

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23
Q

embryology: duodenum distal to ampulla, small bowel, and large bowel to distal 1/3 of transverse colon

A

midgut

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24
Q

distal 1/3 of transverse colon to anal canal

A

hindgut

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25
Q

how does the midgut develop?

A

midgut rotates 270 degrees counterclockwise normally

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26
Q

low birth weight

A
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27
Q

tx: congenital cystic adenoid malformation

A

lobectomy

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28
Q

immunity at birth

A

IgA from mother’s milk

IgG crosses the placenta

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29
Q

1 cause of childhood death

A

trauma

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30
Q

trauma bolus

A

20cc/kg x 2, then give blood 10 cc/kg

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31
Q

tx: bronchiogenic cyst

A

resect cyst

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32
Q

most common mediastinal tumor in children; usually located posteriorly

A

neurogenic tumors (neurofibroma, neuroganglioma, neuroblastoma)

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33
Q

GFR in children (

A

children (

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34
Q

what causes bone growth in children?

A

increased alkaline phosphatase in children compared with adults

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35
Q

what are the umbilical vessels?

A

2 arteries and 1 vein

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36
Q

maintenance IV fluids

A

4cc/kg/hr for first 10 kg
2cc/kg/hr for 2nd 10 kg
1cc/kg/hr for everything after that

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37
Q

four kinds of congenital cystic disease of the lung

A
  • pulmonary sequestration
  • congenital lobar overinflation
  • congenital cystic adenoid malformation
  • bronchiogenic cyst
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38
Q

is thymoma rare in children?

A

yes, thymoma is rare in children

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39
Q

pulmonary sequestration: more likely to have systemic venous drainage (azygous system)

A

extra-lobar pulmonary sequestration

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40
Q

pulmonary sequestration: more likely to have pulmonary vein drainage

A

intra-lobar pulmonary sequestration

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41
Q

how does pulmonary sequestration present? (congenital cystic disease of the lung)

A

most commonly presents with infection; can also have respiratory compromise or an abnormal CXR

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42
Q

tx: pulmonary sequestration (congenital cystic disease of the lung)

A

lobectomy

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43
Q
  • cartilage fails to develop in bronchus, leading to air trapping with expiration
  • vascular supply and other lobes are normal (except compressed by hyper inflated lobe)
A

congenital lobe overinflation (emphysema)

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44
Q

why can hemodynamic instability or respiratory compromise develop in congenital lobar overinflation (emphysema)?

A

same mechanism as tension PTX - vascular supply is compressed by hyper inflated lobe

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45
Q

most commonly affected lobe in congenital lobar overinflation

A

LUL most commonly affected

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46
Q

tx: congenital lobar overinflation (emphysema)

A

lobectomy

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47
Q
  • communicates with airway
  • alveolar structure is poorly developed, although lung tissue is present
  • symptoms: respiratory compromise or recurrent infection
A

congenital cystic adenoid malformation

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48
Q

tx: congenital cystic adenoid malformation

A

lobectomy

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49
Q
  • most common cysts of the mediastinum; usually posterior to the carina
  • are extra-pulmonary cysts formed from bronchial tissue and cartilage wall
  • occasionally are intra-pulmonary
A

bronchiogenic cyst

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50
Q

how do bronchiogenic cysts present?

A

usually present with a mediastinal mass filled with milky fluid

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51
Q

what problems can bronchiogenic cysts cause?

A

can compress adjacent structures or become infected; have malignant potential

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52
Q

tx: bronchiogenic cyst

A

resect cyst

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53
Q

most common mediastinal tumor in children; usually located posteriorly

A

neurogenic tumors (neurofibroma, neuroganglioma, neuroblastoma)

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54
Q

symptoms common to all mediastinal masses regardless of location

A

respiratory symptoms, dysphagia

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55
Q

anterior mediastinal masses

A

T cell lymphoma, teratoma, and other germ cell tumors (most common type of anterior mediastinal mad in children), thyroidCA

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56
Q

tx: cystic hygroma (lymphangioma)

A

resection

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57
Q

middle mediastinal masses

A

T cell lymphoma, teratoma, cyst (cardiogenic or bronchiogenic)

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58
Q

posterior mediastinal masses

A

t cell lymphoma, neuroblastoma, neurogenic tumor

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59
Q

is thymoma rare in children?

A

yes, thymoma is rare in children

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60
Q

caused by reflux of pancreatic enzymes into the biliary system in utero

A

choledochal cyst

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61
Q

why do choledochal cysts need to be resected in children?

A

risk of cholangiocarcinoma, pancreatitis, cholangitis, and obstructive jaundice

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62
Q

choledochal cyst: (85%): fusiform dilation of entire common bile duct, mildly dilated common hepatic duct, normal intrahepatic ducts

A

type 1 choledochal cysts

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63
Q

tx: choledochal cyst: type 1

A

resection, hepaticojejunostomy

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64
Q

choledochal cyst: (3%) a true diverticulum that hangs off the common bile duct

A

type 2 choledochal cyst

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65
Q

tx: type 2 choledochal cyst

A

resection off common bile duct; may be able to preserve common bile duct and avoid hepaticojejunostomy

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66
Q

choledochal cyst: (1%) dilation of distal intramural common bile duct; involves sphincter of Oddi

A

type 3 choledochal cyst

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67
Q

tx: type 3 choledochal cyst

A

resection, choledochojejunostomy

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68
Q

choledochal cyst: (10%) multiple cysts, both intrahepatic and extrahepatic

A

type 4 choledochal cyst

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69
Q

tx: type 4 choledochal cyst

A

resection; may need liver lobectomy; possible TXP

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70
Q

choledochal cyst: (1%) Caroli’s disease: intrahepatic cysts; get hepatic fibrosis; may be associated with congenital hepatic fibrosis and medullary sponge kidney

A

type 5 choledochal cyst

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71
Q

tx: type 5 choledochal cyst

A

resection; may need lobectomy; possible liver TXP

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72
Q

usual causes of lymphadenopathy

A

acute suppurative adenitis associated with URI or pharyngitis

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73
Q

management: fluctuant lymphadenopathy

A

FNA, culture and sensitivity, and antibiotics; may need incision and drainage if it fails to resolve

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74
Q

lymphadenopathy: chronic causes

A

cat scratch fever, atypical mycoplasma

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75
Q

management: asymptomatic lymphadenopathy

A

antibiotics for 10 days -> excisional biopsy if no improvement. this is lymphoma until proven otherwise

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76
Q

lymphadenopathy: usually found in lateral cervical regions in neck; gets infected; is usually lateral to the SCM

A

cystic hygroma (lymphangioma)

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77
Q

tx: cystic hygroma (lymphangioma)

A

resection

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78
Q
  • overall survival 50%
  • increased on left side (80%); can have severe pulmonary HTN
  • diagnosis can be made with prenatal ultrasound
  • symptoms: respiratory distress
A

diaphragmatic hernias and chest wall

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79
Q

are there associated anomalies with diaphragmatic hernias and chest wall?

A

80% have associated anomalies (cardiac and neural tube defects mostly; malrotation)

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80
Q

cxr: diaphragmatic hernia

A

bowel in chest

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81
Q

tx: diaphragmatic hernia

A

high-frequency ventilation; inhaled nitric oxide; may need ECMO

  • stabilize these patients before operation
  • need to reduce bowel and repair defect +/ mesh (abdominal approach)
  • look for visceral anomalies (runt he bowel)
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82
Q

diaphragmatic hernia: most common, located posteriorly

A

bochdalek’s hernia

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83
Q

diaphragmatic hernia: rare, located anteriorly

A

morgagni’s hernia

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84
Q

management: pectus excavatum (sinks in)

A

sternal osteotomy, need strut; performed if causing respiratory symptoms or emotional stress

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85
Q

management: pectus carinatum (pigeon chest)

A

strut not necessary; repair for emotional stress

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86
Q

what can branchial cleft cyst cause?

A

leads to cysts, sinuses, and fistulas

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87
Q

branchial cleft cyst: angle of mandible; may connect with external auditory canal. often associated with facial nerve

A

1st branchial cleft cyst

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88
Q

branchial cleft cyst: on anterior border of mid-SCM muscle. goes through carotid bifurcation into tonsillar pillar

A

2nd branchial cleft cyst

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89
Q

branchial cleft cyst: lower neck, medial to or through the lower SCM

A

3rd branchial cleft cyst

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90
Q

most common branchial cleft cyst

A

2nd branchial cleft cyst

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91
Q

tx for all branchial cysts

A

resection

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92
Q

what causes thyroglossal duct cyst?

A

from the descent of the thyroid gland from the foramen cecum

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93
Q
  • may be only thyroid tissue patient has
  • presents as a midline cervical mass
  • goes through hyoid bone
A

thyroglossal duct cyst

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94
Q

tx: thyroglossal duct cyst

A

excision of cyst, tract, and hyoid bone (At least the central portion)

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95
Q
  • appears at birth or shortly after

- rapid growth during first 6-12 months of life, then begins to involute

A

hemangioma

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96
Q

tx: hemangioma

A

observation - most resolve by age 7-8

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97
Q

does wilms tumor metastasize?

A

frequent metastases to bone and lung

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98
Q

1 solid abdominal malignancy in children

A

neuroblastoma

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99
Q
  • usually presents as an asymptomatic mass

- can have secretory diarrhea, raccoon eyes (orbital metastases), HTN, and opsomyoclonus syndrome (unsteady gait)

A

neuroblastoma

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100
Q

where do most neuroblastoma occur?

A

most often on adrenals; can occur anywhere along the sympathetic chain

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101
Q

when do most neuroblastoma present?

A

most common in 1st 2 yeas of life. children

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102
Q

neuroblastoma is derived from what?

A

derived from neural crest cells

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103
Q

lab findings in neuroblastoma

A

most have increased catecholamines, VMA, HVA, and metanephrines (HTN)

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104
Q

how does neuroblastoma affect vasculature?

A

encases vasculature rather than invades

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105
Q

rare metastases of neuroblastoma

A

go to lung and bone

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106
Q

abdominal xr: neuroblastoma

A

may have stippled calcifications in the tumor

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107
Q

wilms tumor: stage 5

A

bilateral renal involvement

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108
Q

neuroblastoma: what is increased in all patients with metastases?

A

NSE

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109
Q

tx: neuroblastoma

A

resection (Adrenal gland and kidney take; 40% cured)

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110
Q

management of unresectable tumors in neuroblastoma

A

initially unresectable tumors may be resectable after doxorubicin-based chemo

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111
Q

neuroblastoma: stage 1

A

localized, complete excision

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112
Q

best prognosis in hepatoblastoma

A

fetal histology

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113
Q

neuroblastoma: stage 3

A

crosses midline +/ regional nodes

114
Q

neuroblastoma: stage 4

A

distant metastases (nodes or solid organ)

115
Q

neuroblastoma: stage 4-S

A

localized tumor with distant metastases

116
Q

usually presents as asymptomatic mass; can have hematuria or HTN; 10% bilateral
- mean age at diagnosis: 3 years

A

wilms tumor (nephroblastoma)

117
Q

what is prognosis of wilms tumor based on?

A

prognosis based on tumor grade (anaplastic and sarcomatous variations have worse prognosis)

118
Q

does wilms tumor metastasize?

A

frequent metastases to bone and lung

119
Q

management of pulmonary mets in wilms tumor?

A

can resect pulmonary mets if resectable

120
Q

abdominal ct: wilms tumors

A

replacement of renal parenchyma and not displacement (differentiates it from neuroblastoma)

121
Q

how do you differentiate neuroblastoma and nephroblastoma on ct?

A

replacement of parenchyma and not displacement in nephroblastoma

122
Q

tx: wilms tumor (nephroblastoma)

A

nephrectomy (90% cured)

  • if venous extension occurs in the renal vein, the tumor can be extracted from the vein
  • need to examine the contralateral kidney and look for peritoneal implants
  • avoid rupture of tumor with resection, which will increase stage
123
Q

chemotherapy regimen for nephroblastoma (wilms tumor)

A

actinomycin and vincristine based chemo in all unless stage 1 and

124
Q

wilms tumor: stage 1

A

limited to kidney, completed excised

125
Q

wilms tumor: stage 2

A

beyond kidney but completely excised

126
Q

upper GIB 1 year to adult

A

esophageal varices, esophagitis

127
Q

wilms tumor: stage 4

A

hematogenous metastases

128
Q

wilms tumor: stage 5

A

bilateral renal involvement

129
Q

most common malignant liver tumor in children; increased AFP in 90%

A

hepatoblastoma

130
Q
  • fractures, precocious puberty (from beta-hcg release)
  • better prognosis than hepatocellular CA
  • can be pedunculated; vascular invasion common
A

hepatoblastoma

131
Q

tx: hepatoblastoma

A

resection optimal; otherwise doxorubicin and cisplatin based chemotherapy -> may downstage tumors and make them resectable

132
Q

what is survival based on in hepatoblastoma?

A

survival is primarily related to resectability

133
Q

best prognosis in hepatoblastoma

A

fetal histology

134
Q

1 children’s malignancy overall

A

leukemia (ALL)

135
Q

1 solid tumor class

A

CNS tumors

136
Q

1 general surgery tumor

A

neuroblastoma

137
Q

1 general surgery tumor in child

A

neuroblastoma

138
Q

1 general surgery tumor in children > 2 years

A

wilms tumor

139
Q

1 cause of duodenal obstruction in newborns (

A

duodenal atresia

140
Q

1 cause of duodenal obstruction after newborn period (>1 week) and overall

A

malrotation

141
Q

1 cause of colon obstruction

A

hirschsprung’s disease

142
Q

1 liver tumor in children

A

hepatoblastoma; 2/3 of liver tumors in children are malignant

143
Q

1 lung tumor in children

A

carcinoid

144
Q

1 cause painful lower GIB

A

benign anorectal lesions (fissures, etc)

145
Q

1 cause painless lower GIB

A

meckel’s diverticulum

146
Q

upper GIB 0-1 year

A

gastritis, esophagitis

147
Q

what do you think about in adults presenting with intussusception?

A

patient most likely has malignant lead point (i.e. colon ca in cecum) -> OR for resection

148
Q

found on anti mesenteric border of small bowel

  • embryology: persistent vitelline duct
  • # 1 cause of painless lower GIB in children
A

meckel’s diverticulum

149
Q

rule of 2s in meckel’s diverticulum

A

2 ft from ileocecal vale, 2% population, 2% asymptomatic, 2 tissue types (pancreatic - most common; gastric - most likely to be symptomatic) and 2 presentations (diverticulitis and bleeding)

150
Q

dx: meckel’s diverticulum

A

can get meckel’s diverticulum scan with pertechnetate if suspicious of meckel’s diverticulum and having trouble locating

151
Q

tx: meckel’s diverticulum

A

resection with symptoms, suspicion of gastric mucosa, or narrow neck
- diverticulitis involving th ebase or if the base is > 1/3 the size of the bowel, need to perform segmental resection

152
Q

3-12 weeks, firstborn males
projectile vomiting
can feel oliver mass in stomach

A

pyloric stenosis

153
Q

acid base in pyloric stenosis

A

hypochloremic, hypokalemia, metabolic alkalosis

154
Q

ultrasound in pyloric stenosis

A

pylorus >/ 4mm thick

>/ 14 mm long

155
Q

tx: duodenal atresia

A

resuscitation, duodenuoduodenostomy or duodenojejunostomy

156
Q

why avoid fluid resuscitation with K containing fluids in children with severe dehydration?

A

hyperkalemia can develop quickly

157
Q

why avoid non salt containing solutions in infants?

A

hyponatremia can quickly develop

158
Q

why do infants need a maintenance fluid with glucose?

A

because of their limited reserves for gluconeogenesis and vulnerability for hypogylcemia

159
Q

tx: pyloric stenosis

A

pyloromyotomy (RUQ incision; proximal extent should be the circular muscles of the stomach)

160
Q
  • usually 3 months to 3 years
  • currant jelly stools (from vascular congestion, not an indication for resection), sausage mass, abdominal distention, RUQ pain, and vomiting
  • invagination of one loop of intestine into another
A

intussusception

161
Q

intussusception: lead points in children

A

enlarged peyer’s patches (#1), lymphoma, and meckel’s diverticulum

162
Q

rate of recurrence after reduction in intussusception

A

15%

163
Q

tx: intussusception

A

reduce with air-contrast enema -80% successful; no surgery required if reduced

164
Q

intussusception: max pressure with air-contrast enema

A

120 mmHg

165
Q

intussusception: max column height with barium enema

A

1 meter (3 feet)

166
Q

intussusception: high perforation risk beyond 120 mmHg air contrast enema and 1 meter height barium enema

A

need to go to OR if you have reached these values

167
Q

when must you go to OR for intussusception?

A
  • risk of perforation with air contrast or barium enema
  • peritonitis or free air or if unable to reduce

when reducing in OR, do not place traction on proximal limb of bowel, need to apply pressure to the distal limb. usually do not require resection unless associated with lead point.

168
Q

what do you think about in adults presenting with intussusception?

A

patient most likely has malignant lead point (i.e. colon ca in cecum) -> OR for resection

169
Q
  • develop as a result of intrauterine vascular accidents
  • symptoms: bilious emesis, distention; most do not pass meconium
  • more common in jejunum; can be multiple
A

intestinal atresia

170
Q

dx: intestinal atresia

A

get rectal biopsy to r/o hirschsprung’s before surgery

171
Q

tx: intestinal atresia

A

resection

172
Q

1 cause of duodenal obstruction in newborns (

A

duodenal atresia

173
Q
  • usually distal to ampulla of Vater and causes bilious vomiting, feeding intolerance
  • associated with polyhydramnios in mother
  • a/w cardiac, renal, and other gi anomalies
A

duodenal atresia

174
Q

why check chromosomal studies in duodenal atresia?

A

20% of these patients have Down’s syndrome

175
Q

abdominal xr: duodenal atresia

A

shows double-bubble sign

176
Q

tx: duodenal atresia

A

resuscitation, duodenuoduodenostomy or duodenojejunostomy

177
Q

most common type tracheoesophageal fistulas (TEF)

A

type c: most common type (85%)

178
Q

define type c tracheoesophageal fistula

A

proximal esophageal atresia (blind pouch) and distal TE fistula

179
Q

symptoms: newborn spits up feeds, has excessive drooling and respiratory symptoms with feeding; cannot place NGT in stomach

A

tracheoesophageal fistula: type C, type A

180
Q

abdominal xr: TE fistula type c

A

distended, gas-filled stomach

181
Q

define type a TE fistula

A

esophageal atresia and no fistula

182
Q

mc types of TE fistula

A
  • type c (MC - 85%)

- type a (2nd MC - 5%)

183
Q

abdominal xr: type a te fistula

A

patients have gases abdomen

184
Q

define VACTERL syndrome

A

vertebral, anorectal (imperforate anus), cardiac, TE fistula, radius/renal, and limb abnormalities

185
Q

tx: te fistulas

A

right expleural thoracotomy for most perform primary repair, and place g-tube
- azygous vein needs to be divided

186
Q

tx te fistula for infants that are premature,

A

replogle tube, treat respiratory symptoms; place g-tube, delay repair

187
Q

te fistula: complications of repair

A

GERD, leak, empyema, stricutre, and fistula

188
Q

what is survival related to in te fistula?

A

survival related to birth weight and associated anomalies

189
Q

malrotation: symptoms

A

sudden onset of bilious vomiting (ladd’s bands cause duodenal obstruction, coming out from the right retroperitoneum)

190
Q

what is volvulus associated with in malrotation?

A

volvulus associated with compromise of the SMA, leading to infarction of the intestine

191
Q

what causes malrotation?

A

failure of normal counterclockwise rotation (270 degrees)

192
Q

when does malrotation present?

A

75% in the first month, 90% present by 1 year of age

193
Q

management of any child with bilious vomiting

A

needs an UGI to rule out malrotation

194
Q

dx: malrotation

A

UGI - duodenum does not cross midline; duodenal-jejunal junction displaced to the right

195
Q

tx: malrotation

A

resect lady’s bands, counterclockwise rotation (may require multiple turns), place ceum in LLQ (Cecopexy), place duodenum in RUQ and appendectomy

196
Q

causes distal ileal obstruction, abdominal distention, bilious vomiting, and distended loops of bowel

A

meconium ileus

197
Q

rate of meconium ileus in cystic fibrosis

A

occurs in 10% of children with cystic fibrosis

- need sweat chloride test or PCR for Cl channel defect

198
Q

abdominal XR: meconium ileus

A

dilated loops of small bowel without air-fluid levels (because the meconium is too thick to separate from bowel wall); can have ground glass or soapsuds appearance

199
Q

complications of meconium ileus requiring laparotomy

A

can cause perforation, leading to meconium pseudocyst or free perforation

200
Q

tx: meconium ileus

A 
gastrograffin enema (effective in 80%); can also make the diagnosis and potentially treat the patient
- can also use n-acetylcysteine enema
201
Q

surgical management (if necessary) in meconium ileus

A

if surgery required, manual decompression and create a vent for n-acetylcysteine antegrade enemas

202
Q

clasically presents with bloody stools after 1st feeding in premature neonate

A

necrotizing enterocolitis

203
Q

risk factors: necrotizing enterocolitis

A

prematurity, hypoxia, sepsis

204
Q

symptoms: lethargy, respiratory decompensation, abdominal distention, vomiting, blood per rectum

A

necrotizing enterocolitis

205
Q

abdominal xr: necrotizing enterocolitis

A

may show pneumatosis intestinalis, free air, or portal vein air

206
Q

what do you need for monitoring in necrotizing enterocolitis?

A

need serial lateral decubitus films to look for perforation

207
Q

initial tx: necrotizing enterocolitis

A

resuscitation, NPO, antibiotics, TPN, and orogastric tube

208
Q

indications for operation: necrotizing enterocolitis

A

free air, peritonitis, clinical deterioration -> resect dead bowel and bring up ostomies

209
Q

why do you need barium contrast enema before taking down ostomies in necrotizing enterocolitis?

A

to rule out distal obstruction from stenosis

210
Q

mortality in necrotizing entercolitis

A

10%

211
Q

indications for surgery in congenital vascular malformation

A

hemorrhage, ischemia, CHF, nonbleeding ulcers, functional impairment, or limb-length discrepancy

212
Q

tx: congenital vascular malformation

A

embolization (may be sufficient on its own) and/or resection

213
Q

male vs female: imperforate anus

A

more common in males

214
Q

what do you need to check for in imperforate anus?

A

check for associated renal, cardiac, and vertebral (VACTERL) anomalies

215
Q

tx: high (above levators) imperforate anus (meconium in urine or vagina - fistula to bladder/vagina/prostatic urethra)

A

colostomy, later anal reconstruction with posterior sagittal anoplasty

216
Q

tx: low (below levators) imperforate anus - meconium to perineal skin

A

perform posterior sagittal anoplasty (pull anus down into sphincter mechanism); no colostomy needed

217
Q

post op management of imperforate anus

A

need post op anal dilatation to avoid stricture; these patients are prone to constipation

218
Q

intrauterine rupture of umbilical vein; does not have a peritoneal sac

  • decreased congenital anomalies (only 10%) except malrotation
  • to the right of midline, no peritoneal sac, stiff bowel from exposure to amniotic fluid
A

gastroschisis

219
Q

tx: gastroschisis

A

initially place saline-soaked gauzes and resuscitate the patient; can lose a lot of fluid from the exposure bowel; TPN; NPO.

  • repair when patient is stable
  • at operation, try to place bowel back in abdomen, may need vicryl mesh silo
  • primary closure at a later date if silo used
220
Q

failure of embryonal development; has peritoneal sac with cord attached

  • increased congenital anomalies (50%); midline defect
  • sac can contain intra-abdominal structures other than bowel (liver, spleen, etc)
A

omphalocele

221
Q

cantrell pentalogy

A
  • cardiac defects
  • pericardium defects (usually at diaphragmatic pericardium)
  • sternal cleft or absence of lower sternum
  • diaphragmatic septum transversum absence
  • omphalocele
222
Q

tx: omphalocele

A

initially place saline-soaked gauzes and resuscitate the patient; can lose a lot of fluid from the exposed bowel; TPN; NPO

  • repair when patient is stable
  • at operation, try to place bowel back in abdomen, may need vicryl mesh silo
  • primary closure at a later date if silo used
223
Q

what has a better prognosis: gastroschisis vs omphalocele

A

omphaloceleworse overall prognosis compared with gastroschisis secondary to congenital anomalies

224
Q

is malrotation a risk in omphalocele and gastroschisis?

A

yes, in both

225
Q

1 cause of colonic obstruction in infants; more common in males

A

hirschsprung’s disease

226
Q

most common sign of hirschsprung’s

A

infants fail to pass meconium in 1st 24 hours

- can also present in older age groups as chronic constipation (age 2-3)

227
Q
  • get distention; occasionally get colitis

- can get explosive release of watery stool with anorectal exam

A

hirschsprung’s disease

228
Q

dx: hirschsprung’s disease

A

rectal biopsy diagnostic (absence of ganglion cells in myenteric plexus

229
Q

what causes hirschsprung’s disease?

A

is due to failure of the neural crest cells (ganglion cells) to progress in caudad direction

230
Q

what do you need to resect in hirschsprung’s disease?

A

need to resect colon until proximal to where ganglion cells appear

231
Q

tx: hirschsprung’s disease

A

may need to bring up a colostomy initially, eventually connect the colon to the anus (Soave or Duhamel procedure)

232
Q

hirschsprung’s disease: may be rapidly progressive; manifested by abdominal distention and foul-smelling diarrhea
- lethargy and signs of sepsis may be present

A

hirschsprung’s colitis

233
Q

tx: hirschsprung’s colitis

A

rectal irrigation to try and empty colon; may need emergency colectomy

234
Q
  • failure of closure of linea alba; most close by age 3, rare incarceration
  • increased in african americans and premature infants
A

umbilical hernia

235
Q

tx: umbilical hernia

A

surgery if not closed by age 5, incarceration, or if patient has a vp shunt

236
Q

due to persistent processus vaginalis; 3% of infants, M>F

- right in 60%, left in 30%, bilateral in 10%

A

inguinal hernia

237
Q

how can you differentiate hernia from hydrocele?

A

extension of the hernia into the internal ring differentiates hernia from hydrocele

238
Q

tx: inguinal hernia

A

emergent operation if not able to reduce; otherwise, elective repair with high ligation
- consider exploring the contralateral side if left sided, female or child

239
Q

when would you consider exploring the contralateral side in inguinal hernia?

A

if left sided, female or child

240
Q
  • most disappear by 1 year; noncommunicating will resolve; should transilluminate
A

hydrocele

241
Q

tx: hydrocele

A

surgery at 1 year if not resolved or if thought to be communication (Waxing and waning size); resect hydrocele and ligate processus vaginalis

242
Q
  • most common in ileum; often on mesenteric border

- tx: resect cyst

A

cystic duplication

243
Q

mcc of neonatal jaundice requiring surgery

A

biliary atresia

244
Q

jaundice suggesting biliary atresia

A

progressive jaundice persisting > 2 weeks after birth suggests atresia

245
Q

what can biliary atresia involve?

A

can involve either the extra hepatic or intrahepatic biliary tree or both

246
Q

dx: biliary atresia

A

liver biopsy -> periportal fibrosis, bile plugging, eventual cirrhosis
- US and cholangiography can reveal atrocity biliary tree

247
Q

pathway of disease in biliary atresia

A

get cholangitis, continued cirrhosis and eventual hepatic failure

248
Q

what is the Kasai procedure?

A

for biliary atresia - hepaticoportojejunostomy
(1/3 get better, 1/3 go on to liver transplant, 1/3 die)
- involves resecting the extra hepatic bile duct segment

249
Q

when do you need to perform the Kasai procedure?

A

before age 3 months, otherwise get irreversible liver damage

250
Q
  • increased AFP and beta-hcg
  • neonates: sacrococcygeal
  • adolescents: ovarian
  • tx: excision
A

teratoma

251
Q
  • 90% benign at birth (almost all have exophytic component)
  • great potential for malignancy
  • AFP: good marker
A

sacrococcygeal teratomas

252
Q

what is important about the 2 month mark in sacrococcygeal teratomas?

A

2 month mark is a huge transition:

  • usually benign
  • > 2 months -> usually malignant
253
Q

tx: sacrococcygeal teratomas

A

coccygectomy and long-term follow up

254
Q

when do you treat undescended testicles?

A

wait until 2 years old to treat

255
Q

ca risk in undescended testicles

A
  • higher risk of testicular ca in these children
  • cancer risk stays the same even if testicles brought into scrotum
  • get seminoma
256
Q

management of bilateral undescended testicles

A

if undescended bilaterally, get chromosomal studies

257
Q

management of undescended testicles that you cannot feel in the inguinal canal

A

you need to get an mri to confirm their presence

258
Q

tx: undescended testicles

A

orchiopexy through inguinal incision; if not able to get testicles down -> close and wait 6 months and try again; if will not come down, perform division of spermatic vessels

259
Q
  • elliptical, fragmented tracheal rings instead of c-shaped

- wheezing, usually get better after 1-2 years

A

tracheomalacia

260
Q

surgical indication: tracheomalacia

A

dying spell, failure to wean from ventilator, recurrent infections

261
Q

surgery: tracheomalacia

A

aortopexy (aorta sutured to the back of the sternum, open up trachea)

262
Q

most common cause of airway obstruction in infants

A

laryngomalacia

263
Q

symptoms: intermittent respiratory distress and stridor exacerbation in the supine position

A

laryngomalacia

264
Q

what causes laryngomalacia

A

caused by immature epiglottis cartilage with intermittent collapse of the epiglottis airway

265
Q

when do most children outgrow laryngomalacia?

A

most children outgrow this by 12 months

266
Q

is surgical tracheostomy the norm in laryngomalacia?

A

surgical tracheostomy is reserved for a very small number of patients

267
Q
  • obstruction of channel opening (nasal passage) by either bone or mucous membrane, usually unilateral
  • symptoms: intermittent respiratory distress, poor suckling
A

choanal atresia

268
Q

tx: choanal atresia

A

surgical correction

269
Q
  • most common tumor of the pediatric larynx

- frequently involutes after puberty

A

laryngeal papillomatosis

270
Q

tx: laryngeal papillomatosis

A

can treat with endoscopic removal or laser but frequently comes back

271
Q

what is though to cause laryngeal papillomatosis?

A

thought to be caused from hpv in the mother during passage through the birth canal

272
Q

is cerebral palsy associated with GERD?

A

yes, many develop GERD

273
Q

hx: bilious emesis
pe: abdominal distention
dx: plain abdominal film

A

intestinal atresia or stenosis

274
Q

hx: failure to pass meconium
pe: acholic meconium, gastric distention
dx: contrast enema, plain abdominal film

A

duodenal atresia or stenosis

275
Q

hx: bilious emesis
pe: trisomy 21
dx: upper gi contrast study

A

duodenal atresia or stenosis

276
Q

hx: failure to pass meconium
pe: absent anus or visible fistula
dx: plain chest, abdominal film

A

imperforate anus

277
Q

hx: bilious emesis (late)
pe: abdominal distention
dx: ultrasound kidneys, sacrum,r ectum

A

imperforate anus

278
Q

hx: high-risk, premature infant / bilious emesis
pe: abdominal distention / hematochezia, guaiac-positive stool
dx: plain abdominal film

A

necrotizing enterocolitis

279
Q

hx: cystic fibrosis (10%), bilious emesis
pe: acholic meconium, abdominal distention
dx: plain abdominal film, contrast enema

A

meconium ileus

280
Q

hx: bilious emesis, term, healthy infant
pe: no abdominal distention
dx: plain abdominal film, upper gi contrast study

A

malrotation

281
Q

hx: delayed passage of meconium, bilious emesis
pe: abdominal distention, trisomy 21
dx: plain abdominal film, contrast enema

A

hirschsprung’s disease

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